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maι미 Medici굶굶d UfεSciêñ"ce V이.-'O.-Nõτ1(맨랜캔
01，3JAnesthetic management of neurofibromatosis type
1
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ent 8u Hyeon Ha'，
80 Hui Yun'，
Yun 8uk Choi'，
Woo Jin Cho'，
Jong Cook Park'lJeju NationalUniversilvSCh
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1이Medicine~Department이Aneslhesiologyand Pain Medicine.J히u Nalion히UniversitySChool01Medicine，Jeiu，Korea (Received May 8. 2013; Revised May 15. 2013: Accepted May 22. 2013)
Abstract
Neurofibromalosis type 1 is an autosomal dominant neurocutaneous disorder. It is multisystemic disorder 에fh mullilaceled
implications throughoul nearly every organ syslem. Anesthesiologists must perform a through preoperative evaluation of patient More잉'ver lhe anesthesiologist must consider the possibility of each of the multlsyslemic complications when evaluating and
managing Ihe palienllor surgical procedures. (J Med Ufe Sci 2013;10(1 ):44-46)
Key Words : Neurofibromalosis. Anesthesia，Management
Introduction
Neurofibromatosis type 1 is an autosomal dominant inherited disorder characterized by multiple neurofibromas and cafe-aulait spots. Patients with neurofibromatosis type 1 require caution during anestbesia since neurofibromas present ìn ω뼈ue and laryngopharynx could inteñere with
endotracheal intubation or neurofibromas present in the spinal cord could deteriorate pulmonary funcüon by inducing scoliosis or 애phosis
Case Report
A 56-year-old female pati.ent with a height of 158 cm and wei잉lt of 52.6kg was diagnosed with neurofibromatosis
앙pe 1. Since her childhood. t.he patient had some skin lesions similar to a small lump and the lesion at the back did not protrude to cause any trouble in her daily acti.vity However. a skin lesion on the back became bigger enough to be saggy from a week before the hospital visit. She was admitted through the emergency room since bleeding had started from the lower paπ of the lesion (Figure J)‘
Fever was associated with the finding of a decreased hemoglobin level of 7.3 gldL according 1.0 blood test. In addition. she was suspected of the finding of a rapid expansion due to intra-tumor bleeding according to a
Address fα corres
∞
ndence: so HuiYunDepartmenl이Aneslhesiologyand Pain Medlcine，Jeμ Nalional Unive잉i1ySChOOI01Medicire.66 Jejudeahakno.690-756. Jeμ Korea
E-mai1:juguyam@gmait.com
Fi밍ne 1. ÄPIX영rance at the time of admission the patient s
성cin lesion
vascular computed tomography. Cerebral and thoracoabdominal computed tomography and endoscopic examination were conducted preoperatively. No specific fmdings were detected other than the lesion on the back and subcutaneous lesions in the body. However，the patient complained inconvenience of having difficulty in lying on a supine position，daily activity，and particularly sleeping Embolization was peñonned in blood vessels supplying nutrients to the tumors by requesting 1.0 the radiology department one day preφeratively
Since the patient had difficulty in taking a supine position
。
n the day of surgery dueω
the lesion at U1eback. 강te미외blood monitor was placed for the patient after measuring ECG. oxygen saturation. and blood pressure in a later어
decubitus position 끼le patient underwent anesthesia with a
-44-glide scope and a bronchoscope prepared to be used in case
。
f undetected lesions that might result in difficulty in endotracheal intubation. Mter sufficient preoxygenation with 100% oxygen. anesthesia was induced using a 200 mg of thiopental and the ventilation of oxygen mask was checked Subsequently. endotrachea1 intubation、
iVas conducted through a reinforced tube with a inner diameter of 7.0 mm under 1aryngoscopy after administering a 40 mg of rocuronium. Any specific resistance or endotracheal lesions were not obseπed durîng intubation. Although embo1ization was performed in advance，the :MAC씨(Two-lumen centralvenous access set，ARROW) catheter was inserted into the central veno니s line under the induction of u1trasonic waves
in veins of right intemal jugu1ar vein by ta피ng the risk of massive b1eeding into consideration due to the enlarged 1esion. Rocuronium was stea이Jy adminîstered at a 20 mg/hr by maintaining anesthesia at a 20 때Ihr of sevoflurane，a 1.5 1Imin of oxygen，and a 1.5 1Imin of nitrous oxide intraoperatively. Sodium nitroprusside ψas constant1y injected at a 0.5mcg/kg/min to minimize bleeding and systo1ic blood pressure was maintained in between 100-12C mmHg. Mter the patient was comrrmed with norma1îzed voluntary respiration postoperatively ，extubation was conducted in the operating room. Due to the potential risk of unexpected difficulty in breathing and sudden bleeding， she was obseπed in the intensive care unît for three days postoperatively before being transferred to the general ward
f
←←렐윌굵
ion‘Neurofibromatosis type 1 was first documented by
싼'eidrich von Recklinghausen in 1882 and named after the researcher a1so k:nown as von Recklinghausen' s disease， recogrllzed as tumors arising from neπe' tissues. Since neurofibromas might influence both the ectoderm and endoderm ，lesions and associated symptoms cou1d be involved during anesthetic procedure 상U'oughout the body including the central nervous，respîratorγ ，cardiovascular ， musculoske1eta1，digestive ，and genitourinarγ system. Thus， caution is essential with anesthesia. 1n cases where ilifficulty in swallowing，dys따샘ma，voice change ，and other symptoms are particu!arly associated in the aspects of airway management ， p.erforming intubation may be challenging due to lesions in tongue and 1arγngopharynx Since pulmonarγ lesions could induce difficulty in breathing
상 '"ιJ
45
Anesthetie management of neurofibromatosis양pe 1 patient
due to the advancement of pulmonary fibrosis or spinal deforrnity，the presence of such lesions needs to be carefully examined. Patients with a suspected bleeding like the patient in this case report need to be taken int
。
consideration the fact that bleeding may occur due to uíldetected 1esions in gastrointestinal tract[1J. Therefore， anesthetists need to be equipped with anesthètic p1ans and adeqùate countermeasures regarding any potential complications through suffiqient preoperative assessment and historγ taking in all patients[2l Patients "Withneuromuscular diseases are kn。、
m to be sensitive to nondepolarizing muscle relaxants 파m the patient in thîs case' report. For thîs reason，the use of TOF (train of four) or DBS (döub1e burst stimu1ation) is recommended to steadily monitor 야1e neuromuscular system[3l We conducted extubation after confJTIDingthat the patient restored volun잉Iγ breathing and sufficient muscle relaxation of clenchîng her fist for more than five seconds. However，the study had a limitation of insufficient use of the neuromuscular system monitoring that could be taken as an objective indicator. Additional risk factors associated during anesthesia were not detected in preopera미ve examination includîng tracheal lesions，spinal deformity，or lesions in abdominal cavity. Moreover ，she had recovered well in muscle relaxation and minimizing bleedingIt was difficult in maintaining a posture due to neurofibromas. Factors causing trouble in airway management dwing anesthesia were -masses in the neck area and at the back restraining a supine position and requiring caution. Methods enabling anesthesia in a supine position have been introduced by making holes in table for patients who are anticipated to have difficulty in tracheamanagement due to associatêd tracheal deviation during preoperative assessment due to masses at the back[ 4J. Alternative methods were prearranged for the situation of complicated intubation with no anatomical deforrnity in airway management in spîte of unsecured supine position preoperatively. Intubation under bronchoscopy was smoothly performed in lateral position Ultrasonography was used to minimize complications such as
앙terial / puncture or damage in adjacent tissues that could
arise during central venous cannulation due to changed lateral position by safe1y placing centra1 γenous line Therefore，anesthesia was able to be safely induced "Without any particular prob1ems
S니 Hyeon Ha，80 HuÎYnn，Ynn 8uk Choi，Woo Jin Cho，Jong Cook Park
References
1. Hirsch NP，Murphy A，Radcliffe JJ. Neurofihromawsis clînical presentations and anaesthetic implications. Br J Anaesth. 2001; 86: 555-564
2. Fox CJ，Tomajian S，Kaye AJ，Russo S，Abadie JV，Kaye AD. Perioperative management of neurofibromatosîs type 1. Ochsner J. 2012: 12: 111-121
g.
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3. Richardson MG， Setty GK
，
Rawoof SA. Responses to nondepolarizing. neuromuscular blockers and succînylcholine in von Recklinghausen neurofibromatosis Anesth Analg. 1996: 82: 382-3854. Akhlaghi M，Shabanîan G，Abedinzadeh M. Special position for the anaesthetic management of a patient with giant neck and back masses. Ghana Med J. 2010; 44 :37-88
